Michael Liske, MD, FACC, FASE East Tennessee Pediatric Cardiology Knoxville, TN

SUDDEN CARDIAC DEATH

Michael Liske, MD, FACC, FASEEast Tennessee Pediatric Cardiology

Knoxville, TNwww.etpc-hearts.com

“Pistol” Pete Maravich

All time leading division I NCAA scorer with 44 PPG for LSU (would have been 57 PPG with 3 point rule)

"I don't want to play 10 years in the NBA and then die of a heart attack at 40.“ (age 25)

Died at age 40 while playing a pick up game of basketball with James Dobson, 1988

Dx?

“Pistol” Pete Maravich

Single right coronary artery

Flo Hyman

6 ft at 12 years, final height 6 ft, 5 in. In 1984, led the US volleyball team to

Olympic silver 1986: collapsed on the sidelines watching

her team play in Japan Dx?

Flo Hyman

Aortic dissection from Marfan Syndrome “Apart from her height, near-sightedness,

very long arms and large hands, she showed few other physical symptoms. Hyman's sneaker size was a USA size 12.” (Wikipedia)

http://www.knoxnews.com/photos/2009/aug/22/64134/

Jake Logue

Sullivan South (Kingsport) Rebels co-captain and linebacker

Died on the field while playing West High School, Knoxville, August 2009

“Logue had been trailing the play, when he dropped to the ground without being touched. He lay on the field motionless. A fellow South player tried to help him, but Logue was unresponsive.” (News Sentinel)

Dx?

Jake Logue

“A heart problem with his bicuspid aortic valve.” (News Sentinel)

Emmanuel Negedu

Sept 2009: “Collapsed on the practice field before UT trainer Chad Newman and director of sports medicine Jason McVeigh heroically brought Negedu back to life with CPR and an automatic external defibrillator.” (ESPN)

Dr. Stuart Bresee, UT cardiologist, said the initial diagnosis was hypertrophic cardiomyopy, however, “once Negedu quit training, his heart was back to normal, . . the genetic testing was all negative.” (ESPN)

He received an ICD and transferred to New Mexico to continue competitive basketball.

Anyone know what happened to him next?

Emmanuel Negedu

Played 10 games for the U of NM Lobos, and led the team in three games for rebounding

While playing The Citadel in a pre-Christmas game, his defibrillator “produced a reading that led doctors to keep him from finishing the game. He never returned to competition.”SportingNews NCAAB, Apr 2011

Overview

Scope and demographics of the problem Differential diagnoses, including new, in-

vogue conditions Pre-participation screening Practical EKG: normal or abnormal? Rational public health approach Please refer to the 10 questions of your

hand-out!

Scope of the Problem

Maron, registry data of multiple sources (2009): News, LexisNexis, Internet, Center of Catastrophic

Sports Injury, NHLBI pathology archives, direct submissions to registry, etc.

66 sudden cardiovascular deaths per year in USA in athletes age < 39 years, on average.

0.6/100,000 participant-years (vs. 100/100,000 in older adults)

Harmon, sources as above, plus NCAA database (2011): 2.3/100,000 participant-years (NCAA athletes only)

Scope of the Problem, con’t

In a patient with an unrecognized cardiac condition, the risk of SCD is 2.5 times higher if he is an athlete than a non-athlete, Yet, more non-athletes die per year of SCD

because there are more of them. (eg., more non-athletes with HCM die, yet if

you have HCM, your individual chance of death increases substantially if you are an athlete)

These are highly publicized events NOT the most common cause of death in

children

Scope of the Problem

Death in teens age 15-19 years of age

MVA 40% Other accidents 12%

Homocide 14% Suicide 11%Neoplasia 5% CV, non-SCD 1.5%Athlete SCD 1.5% All others 15%

National Vital Statistic Report 2005; Maron-Harmon data

Demographics of SCD(Maron, Circ, 2009)

Males represent 89% of cases. White = 55%, African American = 36%,

Hispanic = 3%, Asian = 1% >50% of HCM deaths are in African

Americans, however, they are much less likely to be identified before they die or have an arrest: Diminished access related to socioeconomic

status? 80% occurred during or immediately after

exertion, 20% at rest.

Demographics of SCD(Maron, Circ, 2009)

Sport(blunt trauma and commotio

cordis excluded)

Basketball 33%Football 25%Soccer 8%All others 34%

Age

High school 59%College 17%Middle school 11%Other 13%

Demographics of SCDWhat is the most dangerous

sport?

Differential Diagnosis: Total(Maron, Circ, 2009)

Differential Diagnosis: Cardiac(AHA Statement, 2007)

Hypertrophic Cardiomyopathy


#1 cause of SCD in competitive athletes General mortality: 1%/yr (higher with some types) Mutations of 11 genes affect cardiac muscle proteins

(sarcomeric proteins) Some forms are inherited AD. Potential Sxs: exertional syncope, palps, CP The murmur is HCM may be soft, but tends to increase

with standing or Valsalva In what other pathologic murmur does this occur? The EKG is abnormal in 95% of cases: ST depression

and T inversion in inferior and lateral precordial leads; prominent q’s, LVH

(EKG normal in 5% of patients)



The degree of septal hypertrophy doesn’t always correlate with the risk of sudden death

Doppler Tissue Imaging may be particularly helpful in borderline cases.

Genetic testing is helpful in some cases. Rx:

Exercise restrictions Meds: if symptoms: blockers and verapamil Septal myectomy: surgical or alcohol ICD in high risk cases (the other two haven’t been

shown to decrease the risk of SCD.)

Congenital coronary anomalies 2nd most common

cause of SCD in athletes

Left coronary arising from right sinus or coronary and coursing between aortic and PA roots is the most dangerous (in 0.15% of pop)

Slit-like orifice and acute angle take off are mechanisms leading to ischemia/arrhythmias.

Congenital coronary anomalies No prodrome in 50% SCD more common in

the young EKG and ETT are

almost always normal Difficult echo

diagnosis in 140 kg linebacker

CT/MRI are accurate Rx:

Exercise restrictions? Prophylactic surgery

is indicated in some cases.

Arrhythmogenic Right Ventricular Cardiomyopathy

#2 cause of athlete SCD in Italy, #4 cause in USA

Mutation of desmosomes (adhesion proteins) between ventricular myocytes

Secondary fibrosis and fatty replacement of myocardium

LV is also involved late Most variants are AD Pathologic and EKG changes

increase with age


EKG clues (difficult diagnosis!): Localized prolonged QRS Epsilon waves in V1 T inversion, especially V2 and V3


Most deaths occur in adulthood (< 15% prior to 18 years)

Difficult diagnosis in childhood; MRI can help

Some gene testing is available

Rx: Exercise restrictions Medical suppression

of arrhythmias ICD

Long QT Syndrome

Most famous “channelopathy” Most LQTS types are due to Na channel “gain of

function” or K channel “loss of function.”

Long QT Syndrome

QTc = QT/sq rt of the preceding RR Top normal = 440 msec in males, up to 450-

460 in teen females EKG normal in 5% of LQTS patients

Long QT Syndrome

Channelopathies as a whole cause only 3% of SCD in athletes

But, . . . , 10% of SIDS Males particularly die early

Long QT Syndrome

Multiple types, each with different genetics (12 so far), presentations, EKG’s and some with varying treatments.

Romano Ward S: heterozygote LQT 1, 2, 3 (>90% of all cases)

Jervell Lange-Nielsen S: homozygote LQT 1, 5; associated with severe sensorineural deafness

Timothy S: LQT8; syndactyly Andersen-Tawil S: also periodic paralysis,

dysmorphisms,

Long QT Syndrome

Torsades de Pointe Prevention and Treatment:

Exercise restrictions Swimming precautions Beta blockers, Na channel blockers Pacemaker if associated with AV block Defibrillation, isoproteronol if bradycardia

related ICD

Long QT Syndrome

Rx, con’t Avoid QT prolonging drugs

www.sads.org

What about all the fuss over Zofran? “Avoid Zofran in patients with congenital long QT syndrome.

ECG monitoring is recommended in patients with electrolyte abnormalities (e.g., hypokalemia or hypomagnesemia), congestive heart failure, bradyarrhythmias or patients taking other medicinal products that lead to QT prolongation.” FDA

http://www.sads.org/

Short QT Syndrome

Another channelopathy

Gain of function of K channels (early repol)

Peaked T’s and shortened QTc, typically < 300 msec

Familial syncope, atrial and ventricular fibrillation

“Impressively lethal”

Short QT Syndrome

ICD’s problematic: with sinus tachycardia, the device interprets peaked T wave as QRS complexes and gives an inappropriate shock

Quinidine has been used

Brugada Syndrome

Another channelopathy

Loss of function of the Na channel that causes LTQ3

EKG (V1 findings) J point elevation ST elevation with a

coved, downward convex pattern.

AD inheritance

Brugada Syndrome

Male:Female = 8:1 Asians Sudden death in sleep

and with bradycardia “Lai Tai” = sleep death,

in Thailand Causes some SIDS Arrhythmias precipitated

by fever in children Rx:

Exercise restrictions (hyperthermia concern)

Quinidine ICD if symptomatic

Others cardiac causes of SCD that might be identifiable

WPW CPVT: Catecholaminergic Polymorphic

Ventricular Tachycardia Idiopathic Ventricular Fibrillation Myocarditis Dilated cardiomyopathy Myocardial bridge over a coronary artery Coronary artery disease Aortic rupture Aortic stenosis MVP – on the list; real disease?

Commotio Cordis

3% of all deaths in athletes Def: sudden death due to low energy

trauma to the chest wall Impact to heart at a critical time of the

cardiac cycle No pre-existing heart disease or genetic

condition, no subsequent rib fractures, homothoraax, or significant myocardial contusion

Primarily age 5-15 years. Pig model, anesthetized animals:

Commotio Cordis

Wooden ball, 30 mph, 16 msec before T wave peak Rhythm?

Commotio Cordis

Wooden ball, 30 mph, on QRS Rhythm?

Commotio Cordis

Implications for our Little Leaguers?

Commotio Cordis 13 year old hit

on chest by inside pitch

Immediate bystander CPR

Passing police car “fortuitous-ly” carried an AED

Normal neurologic outcome

Implications?

Pre-participation Screening

It begins with the PCP! History, history, history! (and physical exam)

EXERTIONAL SYMPTOMS ARE BAD American Heart Association with American

College of Cardiology joint recommendations contain 12 elements (Circ, 2007)

Any positive element should result in a cardiology evaluation.

Tennessee Secondary School Athletic Association form has all 12 elements! (only 7.5 in 2010) – Wonderful!!


What about ancillary testing? What one test will offer more insight at a

lower cost than any other?


EKG (Remember that H and P are critical) When should an EKG be done? On everyone? Just on those with a

positive AHA screen? Is a normal EKG enough to be done with testing after a positive screen?

What about echocardiography?

EKG Testing

The EKG is abnormal in 95% of HCM and LQTS cases; and most cases of WPW, SQTS, pulmonary hypertension, myocarditis, dilated cardiomyopathy, and severe AS. It is abnormal in some cases of Brugada syndrome and ARVC.

BUT, the EKG is typically normal with congenital coronary anomalies, aortic dilation Marfan, some dilated CM, commotio cordis, etc. (altogether 30% of potential cases of SCD)

Diagnosis?High or low risk SCD?








Toward a rational public health approach

In 1982, a mandatory, government funded, annual, pre-participation screening program was instituted for all athletes in Italy, including an H & P with EKG.

Retrospective review of registry and newspapers, Veneto region)

The results were seemingly impressive, with SCD rate reduced to 0.4/100,000 patient-years:


Some have argued that an EKG should be the part of every pre-participation evaluation in the USA.

Barry Maron argues otherwise: US and Italian SCD rates are equal already (2011 Harmon data

would disagree) Can the American sedentary population not be screened?

(most sudden deaths from genetic cardiac disease occurs in non-athletes!)

Cost considerations: False positive cases are much more frequent than true positive

cases, and are COSTLY. The annual cost would be $2 billion (just for the competitive

athlete population) Cost per prevented death = $3.4 million.

The US does not have the physician resources for this program (excess medical students in Italy)


Israeli data, 2011 Mandatory screening, including EKG (yearly) and

ETT (q4 yrs) of all athletes, since 1997. Systematic search of newspaper events (felt to be

inclusive)


So what should we do with conflicting data in the setting of limited resources?

Recall that >50% of HCM SCD’s occur in the African American population, BUT, this population is under-represented in those followed clinically with that condition.

Recall that most SCD’s occur in non-athletes (bigger denominator)

Recall that SCD of athletes represent 1.5% of deaths in teens

For perspective, . . . . . . ,

Scope of the Problem, revisited

Death in teens age 15-19 years of age

MVA, other acci-dents, homocides, suicides 77%Athlete SCD 1.5%All others 22.5%

National Vital Statistic Report 2005, Maron-Harmon Data

Action Steps: ML opinion Do NOW what we know works

AHA/ACC H and P screening of all athletes: “Adherence to uniform guidelines would result in the identification of many more athletes with cardiac disease….” (AHA recs, 2007)

Pediatricians should consider including the elements of the 12 point screen in all well child exams.

Allocate our limited health care funds where we know they will have the greatest impact! The heart is important, but many more kids are dying in accidents, by homicide, and of suicide.

Definitively determine if EKG screening works better than the AHA pre-participation H and P.

And if it does, . . . ,

Summary

SCD is a huge problem for the individual, and so must be taken seriously.

HCM and congenital coronary anomalies are the most common causes of SCD.

The PCP is the key to identifying high risk kids, and a good H & P, including the 12 elements of the AHA screen, are critical.

Screening EKG’s may play a role. Let’s save some lives!

Limited bibliography

Please see your hand-out for a complete list These are highly recommended: Sudden Deaths in Young Competitive Athletes,

Maron et al, Circulation, 2009. ACC/AHA: Recommendations and Considerations

Related to Pre-participation Screening for Cardiovascular Abnormalities in Competitve Athletes: 2007 Update. Maron et al, Circulation, 2007.

Recommendations for Interpretation of 12-lead Electrocardiogram in the Athlete, Corrado et al, European Heart Journal, 2010.

Documents

Michael Liske, MD, FACC, FASE East Tennessee Pediatric Cardiology Knoxville, TN